Uncommon Cause of Acute Adrenal Failure - Case Report

Abstract

Adrenomyeloneuropathy is a rare X-linked inherited disorder of peroxisomes characterized by accumulation of very-long-chain fatty acids (VLCFA) in the central and peripheral nervous system, adrenal glands and testes, leading to dysfunction of these organs and systems (1).   Here, we report a case of adrenomyeloneuropathy presenting initially as acute adrenal crisis, which progressed rapidly within one year to variant neurological manifestations, dementia, sensory, motor and psycho-intellectual dysfunction, and generalized spasticity.